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Novel Procedure for Measuring Psychosine Derivatives by an HPLC Method
Author(s) -
Nozawa Masayo,
Iwamoto Takeo,
Tokoro Toshiharu,
Eto Yoshikatsu
Publication year - 1992
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1992.tb09412.x
Subject(s) - chromatography , high performance liquid chromatography , kidney , leukodystrophy , chemistry , cerebrum , krabbe disease , cerebellum , biochemistry , biology , pathology , central nervous system , endocrinology , medicine , disease
We developed a sensitive and simple method to determine galactosylsphingosine and glucosylsphingosine as a 4‐fluoro‐7‐nitrobenzofurazan autofluorescent compound, using HPLC equipped with a Showdex sugar column. Amounts of galactosylsphingosine were successfully measured in the picomole range. This novel procedure is more stable and simpler than the previous method using o‐phthalaldehyde. It was applied to tissues from the twitcher mouse, an animal model of human globoid cell leukodystrophy. The amount of galactosylsphingosine was 34‐102 μ/kg of wet tissues in control cerebrum and cerebellum, whereas in twitcher mice the range was 2,251‐4,228 μ/kg of wet tissues. The psychosine concentration was also increased in the liver and kidney of twitcher mice, respectively, 1,513 μg; and 1,106 μ/kg of wet tissue (normal liver, 125 μg; normal kidney, 74 μ/kg of wet tissue). This novel procedure is useful for the pathochemical evaluation of lysosphingolipids in various sphingolipidoses as well as in other neuropathological and cellular conditions.